Fundamentals, water, acids, bases and buffers Flashcards
Enzyme deficiency in alkaptonuria
Homogentisate oxidase
Enzyme deficiency in homocystinuria
Cystathionine synthase
Enzyme deficiency in MSUD
Branched-chain alpha-ketoacid dehydrogenase
Enzyme deficiency in PKU
Phenylalanine hydroxylase
Hydroxylation
Introduces an -OH group
Decarboxylation
removes a carboxyl group and release CO2
IN oxidation reactions electrons are ____
IN reduction reactions electrons are ____
Lost
Gained
___% of our body wieght is water
50%
Unique properties of water
Great nucleophile
Great buffer
Hydrogen bonds
Great solvent
pH =
- log [H+]
Hormones that monitor blood volume and osmolality
ADH and aldosterone
Buffers consist of:
Undissosciated acid and its conjugate base
A buffer has its greatest buffering cpacity at:
or near its pKa (+/- 1)
pKa =
- log Ka
Two factors that determine the effectiveness of a buffer
its pKa relative to the pH of a solution
and
its concentration
Norma pH range for blood
7.35 - 7.45
Blood pH is maintained by:
H2CO3/HCO3- buffer system
Ka is:
acid dissociation constant, larger Ka, stronger acid
pKa is:
- log Ka
smaller pKa, stronger acid
Henderson-Hasselbalch equation:
pH = pKa + log [A-]/[HA]
Normal metabolism generates:
CO2, lactic acids and ketones, and inorganic acids
How does the body void of acid?
Expiration of CO2
Excretion of ammonium (NH4+)